Kawasaki Syndrome: Where Are the Answers?

Thirty-six years ago, Tomisaku Kawasaki penned the first description of a quixotic illness that he called mucocutaneous lymph node syndrome.1 Today this illness has been described in almost every country of the world. In studies from North America, Japan, and Western Europe, Kawasaki syndrome has replaced rheumatic fever as the most common cause of acquired heart disease.2 Despite the importance of Kawasaki syndrome as a cause of disease in children, a surprising number of aspects of this syndrome remain ill-defined. Because the etiology remains incompletely understood, the diagnosis is based on history and physical examination. Because the symptoms of Kawasaki syndrome are not unique and diagnosis can be difficult, it is likely that many cases of incomplete or atypical Kawasaki syndrome, as well as some presentations of classic Kawasaki syndrome, remain undiagnosed and untreated, and the child remains at risk of undetected coronary artery disease. Therapy with intravenous immunoglobulin (IGIV) and aspirin, while effective, is nonspecific and is associated with the problems of gamma globulin infusions. These problems include the need for intravenous cannulation, risk of adverse reactions, concern for possible adventitious agents, interference with the immune response to live vaccines, expense, shortages, and the fact that 2% to 4%% of treated children still develop coronary artery disease. Furthermore, the long-term cardiac outcome of children who experience Kawasaki syndrome both for those with and those without detectable coronary artery involvement is not clear. After >3 decades of study, what is known about Kawasaki syndrome and why are there still so many unanswered questions? The study by Holman et al3 in this issue of Pediatrics supports previous estimates regarding certain epidemiologic aspects of Kawasaki syndrome and offers important new detail into others. Using hospital discharge data from the Kids’ Inpatient Database (22 participating states), the authors address a … Address correspondence to H. Cody Meissner, MD, Pediatric Infectious Diseases Division, Tufts-New England Medical Center, 750 Washington St, Boston, MA 02111. E-mail: cmeissner{at}tufts-nemc.org